Carcinoid heart disease induced by a primary ovarian carcinoid tumor is very uncommon but offers the opportunity to study its specific outcome. Indeed, unlike to other carcinoid syndromes that occur in the setting of liver metastasis, a complete removal of the primary carcinoid ovarian tumor is often possible in such cases. We report a case of severe carcinoid heart disease secondary to a carcinoid ovarian tumor. Its interest is related to the exhaustive assessment of the cardiopathy and its complete regression after removal of the ovarian tumor. This outcome emphasizes the need for a as complete as possible reduction of humoral secretions in carcinoid heart disease by medical or surgical therapies before discussing valve replacement surgery.